Anorectal Malformation Management of Female Patients Part II: Pediatric...

All right, just a reminder that a certain percentage, about maybe 2 to 5% of vestibular fistulas have a vaginal septum. So you need to look. I honestly don't believe you need a formal vaginoscopy, but you need to at least look. You need to look into the enteritis, spread with a mosquito or forceps, and prove that there's no septum, because the ideal time to take care of that septum is when the rectum is being mobilized out of the way and the perineal body is open. Many patients have come back years later and the septum was never noticed or touched. And then you have to do a removal of the septum later, later in life. Jack has a comment. There's nothing wrong with that, by the way. You can not touch the septum and then say, please come back after puberty, but you don't want to leave the septum untouched because then this girl will have issues with uh tampons and of course intercourse. Jack. Well, I, I guess my, it's not so much a comment as a question, but our, our gynecologists don't think a septum is a problem. And they, they tell us you don't have to do anything about it. So maybe I can get a comment from, uh, you know what, why don't we hold that because Jerry, Jerry is not here, but she is coming back for the noon session. Oh, is Jerry here? Oh, because you said you were stepping out. You stepped back. OK, we'll get you a microphone. OK. Let's keep, let's keep moving, but, um, let's make sure to talk about, well, answer it, answer it now. Refresh. Because it's. Everyone's thinking about it. If it's time to. And while she's, uh, taking the microphone, you don't think, uh, formal vaginoscopy, uh, is, It's, uh, necessary. You think if there was uh Didelphus you could just see that I think if you look into the perineum and Gerry Pierce, your comment about this too, if you look into the, into the introitus, if you see a septum. There's two hemivaginas. I think at that point you could take out the vaginal scope and see, do you see two normal cervices? Are there, is there mucus at both of them, etc. But if you, but I don't think every single girl needs to be scoped, is my point. Um, because most of them are normal, but if you look in and you see a septum, then you need to recognize that there may be some Eulerian anomaly. Jerry, so the reason that you might, so I would argue that I would give the opportunity, take, take advantage of getting rid of a longitudinal septum. The reason being, oftentimes, you know, these women go undiagnosed, and we see a lot of adult women that oftentimes they are asymptomatic with intercourse, and the women kind of learn the septums and they kind of use one side of the vagina more often than the other one, but oftentimes they're, they're, they're not really bothered with. Intercourse, which is probably why your colleagues are saying you don't have to worry about it. For delivery, if they have a longitudinal vaginal septum, we'll let them labor, and oftentimes they either blow the septum out during labor, which is OK. We resected the time, we repair everything else. It's oftentimes relatively avascular. But the problem is, and women work around this, is tampons. So oftentimes a kid will come in and say, I can't use tampons, or women will say I had to put a tampon in each side, or they'll say I have to use a tampon, but I need a pad. So menstrual hygiene is a huge reason to actually remove it. In my experience with adults that have septums, you know, they're not too terribly bothered with intercourse, and we'll let them labor. I Could you comment on, uh, resecting that septum in an adolescent girl if you, if you do decide to resect it, um, you know, later down the road, uh, how involved is that operation? You know, it's, it's, it's not, it's not hard at all. Yeah, yeah, yes, you can, uh, use anything that has power in it, so, you know, electricity, and you want to get up as much as you can as close to your cervix as you can without damaging your cervix. So the question internationally, so given that, what is the role of vaginoscopy? Um, I, I think, uh, in, in a female perineal or female vestibular, you're obligated to look into the introitus visually with a little spreading of an instrument. Um, and if it's normal, it's normal. Um, if it's abnormal and there is a septum, and maybe Jerry can comment, I think at that point it's very reasonable to define the septum, define the cervix or cervix sees, maybe take out the scope at that point. No, I, I agree. If you have any suspicion in your mind that there might be a septum there, I think it's, it's imperative that the entire length of the vagina is evaluated as well as the presence of cervix or cervix. I will not, uh, today's case is not scheduled for vaginoscopy plus PSA, but I will be looking at the introitis and I will show that to you, hopefully, um, and to see that there's anything there. If we see a septum, we'll take out the vaginoscope, but 97% of these patients are normal. The only point is I think you have to look because you don't want to miss this. You don't want to ignore the possibility that this could be there. The other, um, now, here's a, here's an interesting one. Normal urethra, normal vagina. What is this? A vestibular fistula. B vaginal fistula. What's behind it? A vestibular fistula. B, rectal vaginal fistula. The. And it looks good behind me. This is a, this is a rare picture. You will not see many pictures like this, but I, I, I, Jerry, you would agree that this is within the introitus. This is not a vestibule problem. So what everyone agreed, so this is a, a true vaginal fistula, which is very much like a vestibular but just probably a little bit more mobilization of the rectum. But we don't like the term rectal vaginal fistula because most of them are recto vestibular fistula. But occasionally you may find one like this. OK, we talked a little bit about this before, urethra. Vestibular fistula. Nothing. No vagina. No. Nothing. Right? So this is a vestibular fistula with absent vagina. How about this one? Urethra. Vestibular fistula. Nada. Vestibular fistula with absent vagina, right? OK. Another example. So many, many of these patients have had rectal repairs and the vagina was ignored. And then later in life, there was, uh, so Don, how would you manage the situation if you uh in fact noticed this to be the problem? So, you're going to ask me to go out on a limb again, but I'll tell you what I do. So, the first thing is I think it's really, really, really important to make this diagnosis in a newborn. So every time a newborn is born with imperforated anus, I go to the bedside with the fellows in Q-tips and or the residents in this case, and, and look to make sure there's a vagina there. But assuming there's no vagina, then my preferred method of repair is to use sigmoid colon as a vaginoplasty. So, this would be a case where I might delay the repair, dilate the rectal fistula. And not do a colostomy. Cause if you do a sigmoid colostomy, you are potentially interfering with your blood supply that you would then use to mobilize a sigmoid vaginal graft to do a sigmoid vaginoplasty. And then I would do the repair a little bit older, laparoscopically. In fact, Michael and I did one of these, um, together. And you can use, uh, laparoscopy to harvest a piece of sigmoid and bring it down to the perineum and do the perineal repair all in one stage. So, uh, repair the imperfed anus and do a sigmoid vaginoplasty, um, and then do a backup colostomy. So, um, there are a couple of really interesting features about this anomaly. Um, first of all, the technical, um, um, Don gave one of the options, and that is to do a sigmoid neovagina and use the rectum as rectum, as you would normally do a vestibular or fistula repair. Uh, the alternative approach was to, um, use the, um, rectum as vagina. And immobilize a more proximal portion of the rectum down to be the neo rectum. The downside to that is you're not using rectum as rectum, and the rectum has value for its continence. So I would only do that if I knew that the patient was unlikely to be continent, like they had a spinal anomaly or an absent sacrum. Otherwise I would do exactly as Don described. The other thing we found in this group, and maybe Shammael can get a microphone, is it's a unique group of patients from a urologic point of view. So we presented this back, I think it was, uh, 2009. Of the 33 patients that we had in our series who had, um, absent vagina, 75% of them had urologic problems, including neurogenic bladder. Of that cohort, 50% of them had grade CKD stage 3 or greater. So, solitary kidneys, reflux, hydronephrosis, neurogenic bladder, urinary tract infections are all long-term sequela that have been observed. In this cohort of patients. So, once you make the diagnosis of absent vagina, um, a pretty aggressive screening the urinary tract has to also be employed. So I would add this to the group of patients to be very nervous about and make sure you have urology collaboration. I would say that the male ARM with any associated urologic problem gets a urologic collaboration. Obviously Cloaca, and then vestibular fistula with absent vagina is an important category because a large percentage of those patients had serious urologic things going on with them. Um, this is some technical points here. This is a Patient like that where the urethra is normal. The rectum separates actually very nicely from the urethra. It's a very thick, fibrous type of tissue. It's not nearly as adherent as a rectum is to the posterior vagina, which then allows you to have this space. To then bring through, as Don suggested, your neo vagina. Excuse me, what is the age of surgery in these patients? So what is the age of surgery? So I can tell you if we had a room filled with gynecologists, they would say teenager. But as a pediatric surgeon, well, let's put it this way, what anyone have an opinion about age of vaginal reconstruction? Paula, you have an opinion? Is, is different from us to the urologists and gynecologists. We, we, we tend to do it at the time of the rectal, uh, correction. Yes, so, I think that's the, that is the key point is, um, gynecologists often deal with vaginal anomalies alone, and the rectum and the urethra is fine. So, the timing of that is, is, can we, we can discuss the timing of a Roatansky neovagina. But in the patients that we deal with, we have to fix the rectum. In my opinion, the ideal time to fix the vagina is when you fix the rectum, because you have an open perineal body. It's a great opportunity. I also think from a technical point of view, it's easier when they're younger because that pedicle, this pedicle reaches much easier when the child is young and doesn't have as long a pelvis. So I'd much rather do a neovagina in a younger, uh, in a younger child. This is, this is that neovagina sutured behind the normal urethra, and there's a nice video of that that maybe we can show if there's a gap between, just so you know, there's a file here called Key Videos, and there's a nice video of uh of this case. There's urethra, neo vagina and anus. All right, here's an interesting patient that had a. Um, absent vagina, but normal urethra and normal rectum. And there was this this was a slightly older child filled with blood, and what we did here, normal anus, normal urethra, and through the perineum, and this can also be done laparoscopically, but this one was so close that we were able to open it perineally and make a neovagina. And there's the menstrual blood, and there's the neovagina. So this will, you will see two, normal urethra, normal anus, distal vaginal atresia. And there's your vaginoplasty. All right, so, um, A, a little bit about reoperations. How's this? She needs a. How did the surgeon do here? Looks like that's probably, uh, Uh, no problem. Uh, abnormally located and, uh, may have even had postoperative wound infection there. Looks a little anterior. Yeah, so this is that what we were talking about. This is the urethra, the vagina, and then the dehist uh perineal body. Many, many examples of this. I'm gonna skip through. This is what a cutback looks like, but a cutback in a vestibular. So this does not leave a proper cosmetic result. And here is a anoplasty, but the vaginal septum was ignored. Nothing wrong with that. You just have to go in now and uh remove it. Jerry, do you have a comment as far as timing? Let's say we have this situation. And the child's 6. Would you do it now or would you wait until puberty? I mean, you could You, you caught me talking with Kate about another clinical situation. So here, so here's an anorectal malformation patient. Anus was repaired. Vaginal septum was not diagnosed, and you meet the child at age 6. Yeah, so I don't think unless she's going to sleep for another operation, I don't think that you would need to take her just for this individual procedure to the operating room to fix it. Um, but I think if she's going to sleep for another operation that and you were gonna take advantage of her being asleep already, that'd be reasonable. But prior to that, I don't think there's any rush if you've already sort of missed your window of her primary repair. Um, I don't think there's any rush when she's premonarchal. But you would do it at puberty, at puberty. Only sooner if she was going to be having some other procedure done. Can I just ask something, is the primary determinant. Of whether or not this is a vaginal septum or a double vagina is the primary determinant number of services in terms of your differential diagnosis, meaning, do you, so right, so she could have just a longitudinal vaginal septum with one cervix on top of it, or she could have two, so that's why I think we talked about doing the vaginoscopy to identify what's at the top of the vagina, whether you see one or two cervixs is so important. Um, but they can't have an isolated longitudinal vaginal septum with one malarian system. So we knew, we know that fish have cloacass, but we weren't sure about sharks because they have, um, live births. So we sent Rama to Florida recently. Is Rama here? They're here, so your, um, your assessment was they have cloacas, right? OK. All right. And then the other thing we learned recently, what about whales, Vicky, whales. Chloea or no? Anyone? All right, whales have a cloaca. B, whales do not have a cloaca. No, they shouldn't because A, whales have a cloaca. B, whales do not have a cloaca. They shouldn't maybe. What else don't know. Can, can we send someone? Interesting. Who in person we're gonna send Chris back to do this investigation, right? All right, Vicky, what are people, oh, look at that. Vicky, what's the answer? They do not have a cloaca. That's correct. So they're mammals. See how much knowledge you're getting. OK, nice to see you. So cloaca, single perineal orifice, and we'll end with this, um, and by the way, can you just, what is the, Karen, do you know the lunch is going to be outside as a box and you come back inside? So we need a little time to, to do that, um, um, and then we'll set up for the urology and gynecology portion while you're eating. So I think it's really important to distinguish that there really are two different types of cloacas, the lower ones and the really complicated ones, um, and on the left side is a, uh, artistic diagram of a lower 13 centimeters or less, and on the right side is a picture of the higher 13 centimeter common channel or greater, and I think if you make that distinction you're gonna avoid a lot of trouble. Obviously hydroculpos is a concern, um, and it may in fact obstruct the distal ureters and cause bilateral hydronephrosis. And then about 50% of cloacas have this BIID uh gynecologic system. So here is a newborn, so maybe Michael could describe. How you would manage this newborn female with a cloaca and abdominal mass. So, um, it's most likely a hydroculus. Um, I, uh, Would, uh, do an open, uh, divided colostomy in this particular, uh, patient, and, uh, at the time, I would decompress that vagina with a, uh, pigtail catheter, rather than creating a formal vaginostomy. Um, the, uh, uh, sometimes the, uh, doing a colostomy in these patients can be challenging because that's so dilated. Uh, so, occasionally you have to decompress the vagina, uh, first. And the type of. One mistake, uh, I have made a mistake before trying to, trying to do cystoscopy, vagina, or not, you know, to do, uh, cystoscopy at the time of creating the colostomy, um, in a patient with cloloica and that, uh, makes your, Uh, colostomy creation, uh, very difficult as well. Yeah, I, I, I don't know, uh, if others in the audience have had this experience, but my personal recommendation is to just divert the patient and deal with the vaginostomy and don't worry about scoping them at that time. You have an opportunity to do that at 2 or 3 months of age. It's much more pleasant. You'll get a better, better visualization. There's no rush to scope the vagina at that time. It's important to. What incision? So, um. OK, well, who would do a, uh, a, a left lower quadrant oblique incision? And B, a transverse incision. On a mid transverse or midline transverse or midline. What would. And I'm curious to know how many would approach this laparoscopically. So it's the way that you're going to use it later. So A, left lower quadrant, oblique, B, midline. North or south. So about split. So there was a lovely paper I had the honor of reviewing and the author is in the audience about laparoscopically managing hydroculpos. Would you like to comment? Doctor Speck with the microphone. long enough. OK, well, let's, uh, wait for the microphone so they can hear you out. Doctor Speck is a, uh, Michigan trainee, but now at, now at Vanderbilt. Thank you, Doctor Levitt. Um, Doctor Teitelbaum and I actually at the end of my senior fellow year had this very patient, and we, um, did do a cystoscope and vaginoscopy at the initial, um, portion of the procedure to decompress, and then we put in our laparoscope through a left upper quadrant incision. And we could actually very well visualize all of our pelvic structures. We placed a right lower quadrant vaginostomy tube percutaneously and then a left lower quadrant diverting colostomy. So it's a, it's a nice technique. The alternative approach is through an incision, obviously, and then the judgment of whether to drain with tube or no tube, as Michael alluded to, I would pretty much base on how big the thing is. And if it's a monster hydrocopos and it comfortably reaches the abdominal wall, you can do a a. Sutured, tubeless vaginostomy, and if it's a little bit lower than that, your ideal is to do a tube vaginostomy, but I would suggest using a curled tube rather than a straight tube like a pesser or a Malanot. And I can tell you I learned that the hard way because what happens with the hydroculpos is it recedes from the abdominal wall, the inflammation goes away, and then at about two months, the tube falls out. Whereas if you have a curl tube, that doesn't, uh, that doesn't happen. Dan, do you wanna add anything to your ex fellow's comments? Did she, did she graduate? We, we passed her. Yeah, she's a real attending. OK, good. I, I think she's absolutely right. I think, remember, a lot of this fluid can be vaginal secretions, but a lot of them can be urine reflexing up. And so you, you may wanna, so, so the urine is often a big, as big of a problem as the. Vaginal dilatation. I wanna, I, before you raised your hand, I was gonna call on you guys about the decision about whether to drain the vagina or to drain the bladder. Um. Doctor Curry, please. Mark, I think the issue of putting a tube in the vagina and leaving it in for a couple of months is not the most ideal situation for the baby and the family. One of the things that you may want to try is to have the family just catheterize the cloaca intermittently. And what that does is it drains the vagina and because a lot of, like you were saying, a lot of this fluid is urine after the initial newborn period, the uterus stops secreting and most of the fluid trapped in the vaginal part of the cloaca is urine that's refluxing back into that system because it's a serval mechanism. As the vagina distends, it obstructs the. The urethra and that results in more of the urine leading into the vagina and that keeps that cycle keeps perpetuating itself. So if you just put a catheter in the cloaca 2 or 3 times a day, it drains all that urine and you don't have to in many cases you don't have to do anything with the vagina. I would say that that's a very skin or or put a tube in it. It's a very reasonable approach, but I would add to that that that first, the teaching should be under ultrasound. Because, um, you can have that tube go into the right vagina, the left vagina, the bladder, or the rectum, and you may go 3 days without draining the right vagina, for example. Whereas if you are, uh, under ultrasound showing that the catheter is going where you want it to go, then that's a very reasonable approach. In general, though, you're not draining the structures you want to drain if it's a blind passage through the perineum. Which is why I've advocated for a formal drainage tube or no tube. But if you can get that to work and you prove that the patient is your job is to decompress, as you've said. If you can do that, by all means. Steve, you want to talk about that one case we had that, um, the patient came in very, very sick. Can you give it to Doctor Kraus, the microphone. Patient came in very, very sick and we did a bedside ultrasound. So as Mark said, you have to have a crazy radiologist like me who goes up to the ICU with them and brings my own ultrasound machine to a newborn baby. And I don't know if you have the image, but I, I do have it on my laptop. And it actually shows the very, very dilated hemi vaginas and very echogenic fluid, probably uh meconium and urine, and the patient's creatinine was about 4. And so we went up there. Uh, Doctor Pena put a tube in and I just followed it under ultrasound guidance, and you could do it even in the ICU. Now they have ultrasound machines. You could do it yourself, get the probe, watch the tube go in and see it being drained. And so it's a very nice elegant. One of the things that was very cool about that case is we, when we first did the ultrasound, we could not see the bladder. And then once the hydrocous was drained, the bladder filled beautifully. Which demonstrates the physiology. So the hydrocopos is compressing the ureters and the bladder can't fill. Now once the bladder fills, then the question is, can the urine get out of the bladder? And then on rare occasion you have to drain the bladder too. But I think in most of the patients, you can drain the patient successfully by draining the hydrocopos only. Be aware, like this image shows that there's often a right and left side, so you have to be able to drain both sides, otherwise, only one of the hydronephrosis will, will improve. And, um, this is an image of hydronephrosis before and after drainage of the hydrocopos, and this is what some of these, this is what one of these hydrocopos things looks, looks like. And then what you can do is surgically during the opening of the colostomy is open the dome and then remove a little bit of the septum, and then it becomes a single chamber. All right, I think we're going to quit. I have a question. Please just wait for the microphone. I wonder when if you put a, as Doctor Corey was mentioning, if you put a tube vaginostomy, uh, and you want to do an operation next like couple of months, would it be difficult to bring the vagina because it's fixed not to skin. Can you bring it down? It it would be difficult, because sometimes I find it difficult to bring it down. Yeah. But then, but doing as what Doctor Coley was saying, CIC, mostly it goes through the channel. Yeah, that's true. But if it's one of these very huge hydroculpuses, you're almost always going to need to be in the abdomen anyway, and then you can take down the vaginostomy. I think his way is very good if you can prove that you're in fact emptying the structures. If you're not emptying the structures, you'll have pers persistent hydronephrosis. So, um, there's some more talk about, we hopefully we'll have some time maybe to discuss some of the technical details of Chloe.